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N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV.
Boudewyn, Lauren C; Sikora, Jakub; Kuchar, Ladislav; Ledvinova, Jana; Grishchuk, Yulia; Wang, Shirley L; Dobrenis, Kostantin; Walkley, Steven U.
Affiliation
  • Boudewyn LC; Dominick P. Purpura Dept. of Neuroscience, Rose F. Kennedy Intellectual and Developmental Disabilities Research Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
  • Sikora J; Institute of Inherited Metabolic Disorders, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
  • Kuchar L; Institute of Inherited Metabolic Disorders, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
  • Ledvinova J; Institute of Inherited Metabolic Disorders, 1st Faculty of Medicine, Charles University, Prague, Czech Republic.
  • Grishchuk Y; Center for Genomic Medicine, Massachusetts General Hospital, Harvard Medical School, 185 Cambridge St., Boston, MA 02114, USA.
  • Wang SL; Center for Genomic Medicine, Massachusetts General Hospital, Harvard Medical School, 185 Cambridge St., Boston, MA 02114, USA.
  • Dobrenis K; Dominick P. Purpura Dept. of Neuroscience, Rose F. Kennedy Intellectual and Developmental Disabilities Research Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA.
  • Walkley SU; Dominick P. Purpura Dept. of Neuroscience, Rose F. Kennedy Intellectual and Developmental Disabilities Research Center, Albert Einstein College of Medicine, Bronx, NY 10461, USA. Electronic address: steve.walkley@einstein.yu.edu.
Neurobiol Dis ; 105: 257-270, 2017 Sep.
Article in En | MEDLINE | ID: mdl-28610891

Full text: 1 Database: MEDLINE Therapeutic Methods and Therapies TCIM: Terapias_biologicas / Aromoterapia Main subject: Purkinje Cells / Cerebellum / 1-Deoxynojirimycin / Enzyme Inhibitors / Gliosis / Movement Disorders / Mucolipidoses Type of study: Etiology_studies / Prognostic_studies Language: En Journal: Neurobiol Dis Year: 2017 Type: Article Affiliation country: United States

Full text: 1 Database: MEDLINE Therapeutic Methods and Therapies TCIM: Terapias_biologicas / Aromoterapia Main subject: Purkinje Cells / Cerebellum / 1-Deoxynojirimycin / Enzyme Inhibitors / Gliosis / Movement Disorders / Mucolipidoses Type of study: Etiology_studies / Prognostic_studies Language: En Journal: Neurobiol Dis Year: 2017 Type: Article Affiliation country: United States